Cystic fibrosis. An overview.
A single gene defect in cystic fibrosis results in a spectrum of clinical presentations. Though expression is variable, the radiographic appearance reflects the underlying pathophysiology. Abnormalities of the small airways result in air-trapping; bronchiectasis and mucus plugging reflect large airway disease. These radiographic findings can be assessed and followed using varying scoring systems.
Ruzal-Shapiro
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Department of Radiology, Columbia University, College of Physicians and Surgeons, New York, New York, USA.
D001649:Bile Duct Diseases / Q000503:physiopathology; D001987:Bronchiectasis / Q000000981:diagnostic imaging / Q000503:physiopathology; D003550:Cystic Fibrosis / Q000000981:diagnostic imaging* / Q000235:genetics / Q000503:physiopathology; D010188:Exocrine Pancreatic Insufficiency / Q000503:physiopathology; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000503:physiopathology; D008173:Lung Diseases, Obstructive / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D009093:Mucus; D001261:Pulmonary Atelectasis / Q000000981:diagnostic imaging / Q000503:physiopathology; D014057:Tomography, X-Ray Computed